关键词: Childhood Liver tumor Primary hepatic Burkitt lymphoma Primary hepatic non-Hodgkin lymphoma

Mesh : Male Humans Child Burkitt Lymphoma / surgery diagnosis Prognosis Abdominal Pain Rituximab / therapeutic use Abdomen / pathology

来  源:   DOI:10.1186/s12957-023-03052-3   PDF(Pubmed)

Abstract:
BACKGROUND: Primary hepatic Burkitt lymphoma (PHBL) in children is an extremely rare hepatic malignancy with a dismal prognosis, unless it is detected and treated promptly. An 11-year-old child with abdominal pain was admitted to our hospital. No notable abnormalities were found during his physical examination or laboratory workup, but the abdominal computed tomography and magnetic resonance imaging both indicated a malignant hepatic mass measuring 9.2 × 7.1 × 7.5 cm in size. His postoperative pathology revealed an unexpected primary hepatic Burkitt lymphoma following a laparoscopic liver lobectomy. He then received rituximab and intense multi-agent chemotherapy as treatment. Despite post-chemotherapy bone marrow suppression, the patient eventually made a full recovery and had a good overall state.
CONCLUSIONS: In this study, we describe a rare case of pediatric primary hepatic Burkitt lymphoma and review the literature on clinical features, diagnosis, and treatment for primary hepatic Burkitt lymphoma in children. We stress that this diagnosis should be taken into account in the absence of other single hepatic lesions or primary tumors of hematological disorders, particularly when there is a normal AFP level.
摘要:
背景:儿童原发性肝伯基特淋巴瘤(PHBL)是一种极其罕见的肝脏恶性肿瘤,预后不良,除非它被发现和及时治疗。一名11岁的孩子因腹痛入院。在他的体格检查或实验室检查中没有发现明显的异常,但是腹部计算机断层扫描和磁共振成像均显示大小为9.2×7.1×7.5cm的恶性肝肿块。他的术后病理显示腹腔镜肝叶切除术后出现意外的原发性肝伯基特淋巴瘤。然后,他接受了利妥昔单抗和强烈的多药化疗作为治疗。尽管化疗后骨髓抑制,患者最终完全康复,总体状态良好.
结论:在这项研究中,我们描述了一例罕见的小儿原发性肝伯基特淋巴瘤,并回顾了有关临床特征的文献,诊断,儿童原发性肝伯基特淋巴瘤的治疗。我们强调,在没有其他单个肝脏病变或血液病的原发性肿瘤的情况下,应考虑这种诊断,特别是当AFP水平正常时。
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